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Abstract
Background and Aims Muscular dystrophies are genetic disorders characterized by progressive muscular degeneration. Recommendations for the anesthetic management of these patients frequently include a preference for regional anesthesia and analgesia. Although case reports exist of peripheral nerve blocks in patients with muscular dystrophy, exceptionally few mention important difficulties that may be encountered. We aim to highlight these potential pitfalls.
Methods A thirteen-year-old boy with a diagnosis of Duchenne muscular dystrophy presented for equinovarus foot correction. He had tetraparesis and mild nocturnal hypoventilation. Intravenous anesthesia with target-controlled propofol and remifentanil infusions was combined with ultrasound guided popliteal sciatic and femoral nerve blocks.
A thirty-year-old man with a diagnosis of myotonic muscular dystrophy presented for patellar fracture and ligament reconstruction. He had an intellectual disability, stable hypoventilation syndrome and dysphagia. Ketamine sedation and a subarachnoid block were followed by ultrasound guided popliteal sciatic and femoral nerve blocks, and intraoperative soothing music.
Results Adequate analgesia was obtained without the use of long-acting or postoperative opioids. In the first case, altered muscular echostructure hampered identification of the adductor canal and femoral nerve. Patellar response to nerve stimulation was tenuous even when discomfort was elicited on injection. In the second case, sonographic visualization was unimpeded and nerve stimulation was not used to avoid triggering myotonia. Only light sedation was necessary.
Conclusions Although regional anesthesia is of benefit for patients with muscular dystrophy, potential pitfalls include altered muscular echostructure, a decreased response to or the need to avoid nerve stimulation, and intellectual disability. Expert help should be considered if difficulties are encountered.